Abstract
Problem statement: Amyotrophic Lateral Sclerosis (ALS) is a progressi ve and degenerative disease that affects the anterior horn motor neurons of the spinal cord and pyramidal tracts. In Brazil, there are few epidemiological da ta on this disease. Recently, some important findings have been reported, allowing a better unde rstanding on the underlying processes of neuronal death, as well as the characteristics of t his population. To discuss the clinical and functional profile of a convenience sample of patie nts with ALS in Rio de Janeiro and Neurology Department-Federal Fluminense University to compare the data with studies of other regions and countries. Approach: We used the Severity and Functional Ability Scale (SFAS) as a clinical and functional indicator for ALS. The modified El Escor ial criteria were used to establish the diagnosis. The participants underwent five quarterly assessmen ts during the study period. The research took place at two University Hospitals (Hospital Univers itario Antonio Pedro-Universidade Federal Fluminense and Instituto de Neurologia Deolindo Couto-Universidade Federal do Rio de Janeiro) from March 2007 to December 2009. Results: Of the 98 recruited subjects, only 24 have complet ed all phases of the study. The average age was 52.7±4 .1 years. The time between the onset of first symptoms and seeking care services was 11.6±12.:4 months. The time between the first symptoms and the diagnosis was 20.5±8.4 months. Muscle weakness was identified as the initial symptom in most cases. Patients had impaired muscle strength, speech, swallowing, respiratory function and severity stages of SFAS. The disease had different forms of initial presentation (impaired speech, limbs strength, respiratory function or swallowing) , time to progression and clinical characteristics in our population. Conclusion: The knowledge on the individual clinical evolution in ALS is of paramount importance for the healthcare team to pro vide a correct treatment during the decline of
Highlights
Systematically evaluate multiple aspects of their patients’ health as a way to set more effective interventions and research
The lack of epidemiological data related to motor neuron diseases and especially to Amyotrophic Lateral Sclerosis (ALS) is due to its short evolution time and the inexorable fatal outcome
These factors can be mediated by multiple mechanisms: free radicals, including nitric oxide, intracytoplasmic protein aggregates, Fas/Fas ligand-mediated death pathway, aberrant neurofilament accumulation, glutamatemediated excitotoxicity, abnormal retrograde axonal transport and neurotrophic factor disability (Voltarelli, For inclusion in the study, patients had to present: diagnosis of ALS, probable or probable with laboratory support by the criteria of the World Federation of Neurology (Brooks et al, 2000) and age between 40-85 years
Summary
Evaluation lasted 50 minutes in average and according to the patients’ needs, pauses between 10-15 min breaks. Systematically evaluate multiple aspects of their patients’ health as a way to set more effective interventions and research This process requires a complex understanding of the natural history of the disease as well as an overview of the imposed clinical and functional limitations (Sampaio et al, 2002; Williams et al, 1997). Environmental and endogenous factors seem to contribute to the onset and progression of neurotoxicity in the disease These factors can be mediated by multiple mechanisms: free radicals, including nitric oxide, intracytoplasmic protein aggregates, Fas/Fas ligand-mediated death pathway, aberrant neurofilament accumulation, glutamatemediated excitotoxicity, abnormal retrograde axonal transport and neurotrophic factor disability (Voltarelli, For inclusion in the study, patients had to present: diagnosis of ALS, probable or probable with laboratory support by the criteria of the World Federation of Neurology (revised in 2000-Criteria for EL Escorial) (Brooks et al, 2000) and age between 40-85 years.
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